Segmental arterial mediolysis: Report of 2 cases and review of the literature

Segmental arterial mediolysis (SAM) is an idiopathic noninflammatory vasculopathy involving small to medium arteries, usually in the abdomen, although arteries in the cerebral and coronary circulations also may be affected. Some cases present as abdominal apoplexy due to aneurysmal rupture, but ischemia and infarction also occur. Not uncommonly, SAM may be misdiagnosed as a systemic necrotizing vasculitis. We present 2 patients with bilateral renal infarctions, cerebral arterial dissections, and visceral artery microaneurysms. Both were diagnosed initially as polyarteritis nodosa. The diagnosis was changed to SAM, in one case based on clinical and radiologic features, and in the other, on an open wedge kidney biopsy. We discuss the differential diagnosis and review the literature on SAM.